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Int. J. Mol. Sci. 2016, 17(5), 761; doi:10.3390/ijms17050761

Molecular Mechanisms of Pulmonary Vascular Remodeling in Pulmonary Arterial Hypertension

1
Division of Cardiovascular Medicine, Brigham and Women’s Hospital, Harvard Medical School, Boston, MA 02115, USA
2
Division of Cardiology, Veterans Affairs Boston Healthcare System, Boston, MA 02132, USA
*
Author to whom correspondence should be addressed.
Academic Editor: Anastasia Susie Mihailidou
Received: 7 March 2016 / Revised: 1 April 2016 / Accepted: 8 April 2016 / Published: 18 May 2016
(This article belongs to the Special Issue Molecular Research on Hypertension)
View Full-Text   |   Download PDF [809 KB, uploaded 18 May 2016]   |  

Abstract

Pulmonary arterial hypertension (PAH) is a devastating disease that is precipitated by hypertrophic pulmonary vascular remodeling of distal arterioles to increase pulmonary artery pressure and pulmonary vascular resistance in the absence of left heart, lung parenchymal, or thromboembolic disease. Despite available medical therapy, pulmonary artery remodeling and its attendant hemodynamic consequences result in right ventricular dysfunction, failure, and early death. To limit morbidity and mortality, attention has focused on identifying the cellular and molecular mechanisms underlying aberrant pulmonary artery remodeling to identify pathways for intervention. While there is a well-recognized heritable genetic component to PAH, there is also evidence of other genetic perturbations, including pulmonary vascular cell DNA damage, activation of the DNA damage response, and variations in microRNA expression. These findings likely contribute, in part, to dysregulation of proliferation and apoptosis signaling pathways akin to what is observed in cancer; changes in cellular metabolism, metabolic flux, and mitochondrial function; and endothelial-to-mesenchymal transition as key signaling pathways that promote pulmonary vascular remodeling. This review will highlight recent advances in the field with an emphasis on the aforementioned molecular mechanisms as contributors to the pulmonary vascular disease pathophenotype. View Full-Text
Keywords: pulmonary arterial hypertension; DNA damage; microRNA; metabolism; mitochondria; endothelial-to-mesenchymal transition pulmonary arterial hypertension; DNA damage; microRNA; metabolism; mitochondria; endothelial-to-mesenchymal transition
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Leopold, J.A.; Maron, B.A. Molecular Mechanisms of Pulmonary Vascular Remodeling in Pulmonary Arterial Hypertension. Int. J. Mol. Sci. 2016, 17, 761.

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